How Phineas

It seems like so much happens but there's so little I can report. As my parents say, things change from phone call to phone call. And it's true, it's been a real roller coaster. The major ups and downs lately have had to do with Fin's diagnosis and how to treat him. The doctors in Bangor determined that Fin has tracheobronchomalacia. They performed a bronchoscopy with a flexible scope and it showed pretty serious airway floppiness as well as weak bronchial openings. Basically, unless Fin's own work of breathing keeps his airway open, he has obstructive apnea. This is not a great scenario, obviously, but it is treatable and there are things that can be done to help. But first, the Bangor team wanted the Boston specialists to take a look at him.

So Fin was flown to Boston, where an ENT specialist, a pulmonary specialist, and a cardiologist took a look at him. And while the opinions varied day to day, and his overall condition apparently improved dramatically all by itself, they all agreed that the tracheobronchomalacia appeared to be a major part of his health problems. The question was what to do about it. The best treatment, C-PAP (positive pressure into the airway) is the usual route, but Phineas does not tolerate C-PAP. He always opens his mouth and lets the air out, so it doesn't help ventilate him. Everyone agreed that the thing to do was to bypass the problem, and that meant a tracheostomy.

However, no one could tell us definitively that a trach needed to be done. It could help, but as they kept looking at Fin and going over his numbers, everything just looked so good.... not like a kid that needed a trach. It always came back on me and Jess to decide, and that was a tough call to make. We couldn't see putting a hole in his throat if it wasn't going to fix the overall problem, even if it helped out somewhat in the long run. Besides, as he improved, it looked less and less likely that he needed a trach. He had a few oxygen desaturations overnight on a couple different occasions, but nothing to write home about. It began to look like we might get the specialists two cents and then a trip back home.

Then Fin began to act up. Coughing. A slight temp. Quick desaturations. He was up to his same old tricks. In a way, we were slightly relieved, because it became clear that we didn't end up in Boston for nothing, and they got to see what we have been dealing with for months. Then his blood CO2 began to rise, and the doctors suggested C-PAP. Jess shook her head. She said mark her words; he'd be intubated before the night was over. And she was right. He couldn't clear his secretions or keep his O2 saturation up, and C-PAP didn't help, so they put him on the ventilator at about 1 am.

All this made up everyone's mind. Fin needs a tracheostomy. It will help stent open his airway temporarily and allow for easier ventilation instead of constantly needing a breathing tube. It will allow him to grow and sleep better and keep the CO2 out of his bloodstream. It will feel like a setback, but hopefully, it will allow him to get better long-term. Unfortunately, we have to wait until he's over this respiratory infection that he came down with while we waited to decide (how Phineas) before they can do the tracheostomy. Maybe we waited too long. Maybe all the watching and mulling over the options allowed him to get sick again. Maybe he would have gotten sick anyway. This is his pattern. It feels like a kick in the pants, but it's how Phineas is. As one of our nurse friends said, he'll get there. He just likes to take the long way.

And finally, on June 24th, he had the surgery. He already looks better. For one thing, you can see his whole adorable little face. Go Fin!

Up in the Air

Three days at home.
Dang.

The issue is with Fin's breathing again. He's happy and doing great while he's awake, but as soon as he goes to sleep, the trouble starts. His bronchioles get plugged with mucous and that causes him to work overtime to breathe. The first night at home was fine but the second and third were spent making sure he was getting air. Sometimes, he wasn't. So we took him to the emergency room at 3 am on Sunday.

No one is sure exactly why this happened. One of the doctors said it looked like an asthmatic event, which we sort of expect due to his chronic lung disease. Many people have suggested allergens or irritants in our home air. It could be time to take a serious look at changes in our house. One of the main things we can do is lower the number of pets. This makes the most sense, but it's a hard decision to make, for many reasons. The girls don't want to lose their cats. I don't think we could possibly part with our dog. Part-time, trial-basis relocation of the animals is a possibility. We just aren't sure. For now, we have HEPA filters in two rooms and have replaced the carpet in the living room with a new floor.

After Fin was admitted to PICU, they started doing blood work and respiratory viral tests. The CO2 level in his blood was 117. It should be around 40. They put him on constant nebulizers and he started to look better. He was awake, clapping his hands, and having a grand old time. The doctor whipped out his camera phone and took a picture. He couldn't believe a kid with a CO2 level that high could possibly be laughing and enjoying himself. He wanted to document the moment to confound his medical students. By lunchtime, Fin's heart rate was normal and his CO2 had dropped to 59.

So he went to bed and we went home. In the morning, he was working to get air again and his CO2 level started to climb. He had another rough night trying to breathe and plugging up. His lung X-Ray looked better but he was all junky and wet with thick mucous secretions. They suctioned him constantly and put him back in his little nebulizer tent, this time at a higher dose of medication. He slept fitfully in there, struggling with everything from his chest PT to his medication. Everything indicated that he was fighting something, his CO2 level, bone marrow, heart rate, all the numbers were off. And again, when he woke up, he was happy, smiling, laughing, saying "Dadda". He had a bath and Jess dressed him while they continued to suck yellow junk out of his airway. The kid must really like the hospital or hate his parents, I don't know which. Either way, we need help! Fin needs help! He needs to breathe and sleep!

Fin's Surgery

Phineas had surgery yesterday, May 6th, to reverse his ileostomy and reconnect his small intestine. Everything went well. He looked great before he went into the procedure at 1:30 in the afternoon and he looked great when we saw him back in his room at 6:00 in the evening. He was intubated during the operation but when we saw him, there was no breathing tube, and he was doing fine on his own. The doctor said all the internal tissue looked good and clearing the old scar tissue was the most time-consuming part. But he used the same incision as before and Fin tolerated the whole thing very well.

The only down side is that they were unable to save the ileocecal valve. This is a small sphincter muscle that regulates the flow between the small and large intestine. Without it, Fin will have some digestive issues as he grows and the possibility of bacterial overgrowth and backflow from the large intestine into the small. These things are manageable, but not ideal. We are a little bummed about it, but considering all Fin has been through, it seems a small price to pay.

The next steps are bowel rest for a few days, pain regulation, fever watch, and making sure he's happy and comfy. Phineas is a real trooper and despite the numerous challenges in his ten months of life, he continues to improve. We are so glad he has the fighter's spirit. It has certainly helped him so far!

Back in Bangor :)

After a two week stay in Boston, Phineas was allowed to return to our home hospital. They made some changes, some tweaks in his overall care, and got him over the lung infection and kidney issues without having to use dialysis. Which, of course, made us all extremely happy. The doctors and nurses took good care of Fin and were in frequent contact with the staff in Bangor. They had very good things to say about the team at EMMC, which made us very happy, because they have been fantastic to us and Fin.

The main reason Fin went to Boston was to meet a team of gut specialists and see if they could come up with a plan on his feeding issues. We are happy to report that the prognosis is very good and the next step will be reconnecting his intestines. The overall feeling is that we should get his colon working again and see how things go. He's still getting TPN and might for a while, but he's on track to reverse the ileostomy and start down the road to near-normal babyhood. It's pretty exciting, and we feel good about things, even if he has to remain in the hospital until the day of surgery. The doctors want to make sure he's in a place of good health before they operate again. His kidneys, which took a major hit due to dehydration, might take some more time to normalize, but his response to the treatment has been amazing. It's great to see him looking happy and cute again. I think he's happy to be back in Bangor, too.

The latest......

Boston seems to be a good place for Fin. He's seen a lot of specialists, and they're coming up with a plan to treat him. We feel he's in good hands. So much so, that while the doctors and nurses took care of him, we hit the town with the girls and Mimi and Papa and had a ball! Lots of food, lots of walking, lots of sightseeing. The girls loved the New England Aquarium, and we had so much fun in the city that we felt guilty leaving poor Phineas in the hospital. But it was good for Jess to get out of the same four walls and get some fresh air. Now she knows of a few places she can shop...

There are many issues for Fin to deal with as we move along, but nutrition still remains the number one focus. They are still very optimistic that his gut can eventually return to normal. It will be a process, but there are many eyes watching him, and now we are armed with more information, a better idea of his tolerance for changes, and emergency numbers for the rough patches. As he improves, we'll have more conversations about the plan.

Also, due to the effects of severe dehydration, his overall kidney health is in question. It can take up to 12 weeks for kidneys to normalize after a bout of tubal necrosis, and Fin's kidneys were already compromised in the first place. But the kidneys, especially in little people, are very forgiving and resilient organs, so we don't know where they'll level out again. But that's not the major issue. His gut is the focus, and that's the key to his health as he grows.

But let's focus on now. He had a decent wakeful period today and has shown signs of being ready to come off the vent. His numbers are looking good, he's making good urine, and he's flashing those big, bright eyes and raising his expressive brows at all the nurses. He's also fighting the breathing tube a lot, and thrashing around like a madman. He's a little tired of being stuck in his bed. The plan is to extubate him soon if he doesn't do it himself first. It's hard to keep Fin restrained, and he's not even mobile yet. The kid has some spunk, that's for sure.

Off to Boston!

Looks like we're going to Boston. Fin has kept dumping out his food instead of digesting it, which left him quite dehydrated. Enough so that his kidneys are doing very poorly. He was obviously super dehydrated, so they gave him the appropriate amount of fluid to help him out. He has all this fluid on board now but he's not quite peeing it out. The body's response to being too acidic is to hyperventilate and blow off the carbon dioxide. Well, Fin's not quite doing that either. That's why they put him on the vent to manage his respirations with the machine. And that seems to be working, for now. There's a big balance to try to achieve with Fin's short gut. We have to find the right amount of so much nutrition by his tummy and so much from IV. When we exceeded Fin's limit by gut, he just started pouring out his ileostomy. So when we pulled back, he didn't. The doc described it as making changes while driving 80 mph on ice. We originally came into the hospital on Saturday morning to just get his pH back in balance with adding sodium and stuff. Then we found out he was in acute renal failure. That's the way it seems to work with Phineas. Bring him in for one thing to unearth something more serious. But that's o.k. We feel like he'll be o.k. We'll see what happens!

Progress is Progress

Jess updates:

As it turns out, Phineas will need TPN longer than originally expected. He was scheduled to come off it altogether in a week, but now they are upping his dose. He lost some weight, became quite dehydrated, and his ileostomy output increased. All this leads the doctor to believe that he's not absorbing fluid and food as well as he should, and his nutrition and TPN balance is in question again. He said Phineas has been making progress right along, and while this is a setback, it's not an unexplained one. This is the way it goes for shortish gut kids. It's all about the nutrition. Everything else will fall in line. We need to find the right combination of IV fluid and calories for him to continue growing until they hook his intestines back together. The doctor said we should look at this as fifteen steps forward and just a handful of steps back, since it's amazing Fin grew at all considering he's not using most of his plumbing, including the parts of his colon that absorb fluid.

Fin's also been exploring some foods! He hasn't quite grasped the swallowing and tends to want to protect his airway primarily. But he likes bananas, carrots and oatmeal the most. Hey - progress is progress!"